Pulmonary arterial hypertension

Case contributed by Dr Alexandra Stanislavsky

Presentation

Exertional dyspnoea

Patient Data

Gender: Female
X-ray

There is massive enlargement of the pulmonary trunk and the right and left main pulmonary arteries.
The pulmonary trunk shows delicate mural calcification.

There is massive enlargement of the pulmonary trunk and the right and left main pulmonary arteries with abrupt pruning of the peripheral pulmonary arteries. There is also mural calcification of the pulmonary trunk (best seen on the plain film), in keeping with chronic pulmonary hypertension. Bowing of the interventricular septum to the left.

Case Discussion

This patient has known idiopathic pulmonary hypertension, and her imaging shows well the typical appearances.  

Idiopathic pulmonary arterial hypertension is essentially a diagnosis of exclusion.  The differential is that of causes of secondary pulmonary arterial hypertension and includes among others:

For a more complete list, please refer to the article on causes of pulmonary arterial hypertension.

None of these were present in this patient.

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Case information

rID: 12644
Published: 28th Dec 2010
Last edited: 10th May 2019
System: Chest
Inclusion in quiz mode: Included

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